scholarly journals Chiari Malformation- Report of 3 Cases

2010 ◽  
Vol 23 (1) ◽  
pp. 84-86
Author(s):  
Md Zahed Hossain ◽  
Md Kafil Uddin

3 cases of chiari malformation type-I were presented with classical picture of spasticity, quadriparesis and dissociated sensory loss and were operated ( Craniovertebral junction decompression by midline suboccipital craniectomy with removal of C1 arch and duroplasty). All the 3 patients were improved. Among them one patient recovered with some morbidity for postoperative other diseases. TAJ 2010; 23(1): 84-86

Author(s):  
Ioan Alexandru Florian ◽  
Mihaela Maria Pop ◽  
Teodora Larisa Timis ◽  
Ioan Stefan Florian

Objective: To present an alternative surgical technique in treating cases of Chiari I Malformation with mild-to-moderate syringomyelia after decompressive suboccipital craniectomy: incising only the outer layer of the dura mater, then dissecting it from the inner layer without opening the latter. Methods and Results: We utilized this technique in a short series of three cases who were admitted in our department for mild symptoms such as intermittent headache and dissociated sensory loss in the upper limbs, caused by a Chiari Malformation Type I. The patients were placed in the sitting position. We performed a reduced median suboccipital craniectomy and resection of the posterior arch of C1 adapted to the level of tonsil descent, from a limited superior half to a complete resection. Afterwards, we incised the outer dural layer, while sparing the inner one. Using a fine dissector, we then split apart the outer and inner layers to the margin of the craniectomy. Through the transparency of the inner layer and the arachnoid, the cerebellum and the medulla were visible and pulsating. An autologous fascia duraplasty was then performed. The postoperative course was favorable in all cases, patients being discharged without any deficits and with complete symptom resolution. Conclusions: Interlayer dural split technique can be used effectively in treating symptomatic cases of type I Chiari malformation in adults, with mild-to-moderate syringomyelia. It is less invasive than opening the dura and possibly more effective than decompressive craniectomy and C1 laminectomy alone. This technique must be validated in a larger case-control series.


2021 ◽  
Vol 28 (1) ◽  
pp. 28-33
Author(s):  
Nicholas S. Szuflita ◽  
Tiffany N. Phan ◽  
Jason H. Boulter ◽  
Robert F. Keating ◽  
John S. Myseros

OBJECTIVE The authors aimed to describe the natural history and optimal management of persistent syringomyelia after suboccipital craniectomy for Chiari malformation type I (CM-I). METHODS A cohort of all patients who presented to a tertiary pediatric hospital with newly diagnosed CM-I between 2009 and 2017 was identified. Patients with persistent or worsened syringomyelia were identified on the basis of a retrospective review of medical records and imaging studies. The management of these patients and their clinical courses were then described. RESULTS A total of 153 children with CM-I and syringomyelia were evaluated between 2009 and 2017. Of these, 115 (68.8%) patients underwent surgical intervention: 40 patients underwent posterior fossa decompression (PFD) alone, 43 underwent PFD with duraplasty, and 32 underwent PFD with duraplasty and fourth ventricle stent placement. Eleven (7.19%) patients had increased syringomyelia on subsequent postoperative imaging. Three of these patients underwent revision surgery because of worsening scoliosis or pain, 2 of whom were lost to follow-up, and 4 were managed nonoperatively with close surveillance and serial MRI evaluations. The syringes decreased in size in 3 patients and resolved completely in 1 patient. CONCLUSIONS Persistent or worsened syringomyelia after CM-I decompression is uncommon. In the absence of symptoms, nonoperative management with close observation is safe for patients with persistent syrinx.


2007 ◽  
Vol 7 (4) ◽  
pp. 450-453 ◽  
Author(s):  
Daniel M. Oberer ◽  
Oran S. Aaronson ◽  
Joseph S. Cheng

✓ The authors describe a previously undocumented complication of suboccipital craniectomy combined with duraplasty for the treatment of Chiari malformation Type I and propose techniques to prevent its occurrence. Although there have been reports of epidural pseudomeningoceles in the setting of spontaneous intracranial hypotension and intracranial hygromas following suboccipital craniectomy with duraplasty, the authors believe this case to be the first instance of quadriparesis caused by the delayed formation of a compressive epidural cerebrospinal fluid collection after suboccipital craniectomy with duraplasty. This complication is significant and must be recognized given the potential severity of neurological insult and the number of these procedures performed yearly in both the pediatric and adult populations.


2017 ◽  
Vol 126 (2) ◽  
pp. 626-633 ◽  
Author(s):  
Aintzane Urbizu ◽  
Alex Ferré ◽  
Maria-Antonia Poca ◽  
Alex Rovira ◽  
Juan Sahuquillo ◽  
...  

OBJECTIVETraditionally, Chiari malformation Type I has been related to downward herniation of the cerebellar tonsils as a consequence of an underdeveloped posterior cranial fossa. Although the common symptoms of Chiari malformation Type I are occipital headaches, cervical pain, dizziness, paresthesia, and sensory loss, patients often report symptoms related to pharyngeal dysfunction such as choking, regurgitation, dysphagia, aspiration, chronic cough, and sleep disorders. In addition, tracheal intubation is often difficult in these patients. The purpose of this study was to analyze the morphological features of the oropharynx and oral cavity in patients with Chiari malformation Type I to help identify underlying anatomical anomalies leading to these debilitating symptoms.METHODSSeventy-six adult patients with symptomatic Chiari malformation Type I with cerebellar tonsillar descent greater than 5 mm below the foramen magnum and a small posterior cranial fossa and 49 sex-matched controls were selected to perform a retrospective case-control MRI-based morphometric study in a tertiary hospital. Eleven linear and areal parameters of the oropharyngeal cavity on midsagittal T1-weighted MRI were measured and the average values between patients and control cohorts were compared. Correlations between variables showing or approaching statistical significance in these structures and posterior cranial fossa measurements related with the occipital bone were sought.RESULTSSignificant differences were detected for several oropharynx and oral cavity measures in the patient cohort, primarily involving the length and thickness of the soft palate (p = 9.5E-05 and p = 3.0E-03, respectively). A statistically significant (p < 0.01) moderate correlation between some of these variables and posterior cranial fossa parameters was observed.CONCLUSIONSThe existence of structural oropharyngeal and oral cavity anomalies in patients with Chiari malformation Type I was confirmed, which may contribute to the frequent occurrence of respiratory and deglutitory complications and sleep disorders in this syndrome.


2019 ◽  
Vol 23 (6) ◽  
pp. 704-707 ◽  
Author(s):  
Christopher E. Louie ◽  
Jennifer Hong ◽  
David F. Bauer

Suboccipital craniectomy with duraplasty is a commonly performed procedure for children with symptomatic Chiari malformation type I (CM-I). Several dural substitutes are used for duraplasty, ranging from pericranium to synthetic materials. When available, autologous pericranium is often preferred due to its low cost, performance in obtaining a watertight closure, ease of suturing, and absence of immune reaction. Long-term follow-up data on the durability of various dural substitutes are lacking. The authors report a rare, long-term complication of duraplasty performed using an autologous pericranial graft, and they conduct a literature review of similar complications. Heterotopic ossification of an autologous pericranial graft is a rare complication of duraplasty. This dystrophic bone growth can be symptomatic due to compression of neural structures, and it requires reoperation for removal. Surgeons should consider this rare long-term complication in patients presenting with unusual symptoms after duraplasty with pericranium.


2011 ◽  
Vol 8 (6) ◽  
pp. 620-624 ◽  
Author(s):  
Cordelie E. Witt ◽  
Anthony C. Wang ◽  
Cormac O. Maher ◽  
Khoi D. Than ◽  
Hugh J. L. Garton ◽  
...  

In this report, the authors describe the first known case of inducible hemifacial weakness in a patient with Chiari malformation Type I (CM-I). The patient was a 14-year-old girl with a 1-year history of right facial paresis induced by sustained leftward head rotation. These episodes were characterized by weak activation of her right facial muscles with preserved eye opening and closure. Additionally, she had hypernasal speech, persistent headaches, and intermittent left arm twitching. Magnetic resonance imaging demonstrated a CM-I. A suboccipital craniectomy and C-1 laminectomy were performed for decompression of the CM-I, with duraplasty and coagulation of the pial surface of the cerebellar tonsils. At the 9-month follow-up, the patient's inducible hemifacial weakness had completely resolved. Her symptoms were thought to have resulted from the CM-I, perhaps due to traction on the right facial nerve by the ectopic tonsils with head rotation.


2018 ◽  
Vol 28 (3) ◽  
pp. 262-267 ◽  
Author(s):  
Tatsushi Inoue ◽  
Natsuki Hattori ◽  
Tsukasa Ganaha ◽  
Tadashi Kumai ◽  
Shinichiro Tateyama ◽  
...  

Chiari malformation type I (CM-I) is typically treated with foramen magnum decompression. However, a recent study proposed a new technique for patients with CM-I, wherein only short atlantoaxial joint fusion and distraction is applied. Posterior fusion with or without atlantoaxial distraction is a potential option for patients with CM-I associated with basilar invagination or complex anomalous bony craniovertebral junction pathology, since this procedure allows clinicians to avoid using the technically demanding transoral approach in which some or all of the odontoid tip is invisible. Distraction of the atlantoaxial joint reduces ventral cervicomedullary compression, leading to neurological improvement. Here, the authors report the case of a 50-year-old woman with CM-I plus basilar invagination whose condition immediately improved but then gradually deteriorated following atlantoaxial joint distraction and fusion. Therefore, the authors performed endonasal/transoral odontoidectomy, which resulted in prolonged neurological recovery. Short atlantoaxial fusion with distraction is a smart and ideal surgical planning concept that can result in significant neurological improvement. However, this case suggests that anterior odontoidectomy is still an essential element of the intervention strategy for patients with CM-I with complex craniovertebral junction pathology.


2008 ◽  
Vol 1 (2) ◽  
pp. 118-123 ◽  
Author(s):  
Nicholas M. Wetjen ◽  
John D. Heiss ◽  
Edward H. Oldfield

Object To better understand syrinx pathophysiology, the authors performed a prospective study in which they used findings from serial clinical and magnetic resonance (MR) imaging examinations performed before and after craniocervical decompression to establish the time course of syrinx narrowing. Methods Serial clinical examinations and cervical MR imaging were performed in 29 consecutive patients with Chiari malformation Type I (CM-I) and syringomyelia before surgery, 1 week, and 3–6 months after surgery, and then annually. Time to narrowing of the syrinx (> 50% decrease in maximal anteroposterior diameter) following surgery was calculated using the Kaplan–Meier method. Results All syringes decreased in diameter and length (number of segments) on MR images at 3–6 months, 1 year, and 2 years or later. The syrinx diameter decreased from 6.9 ± 2.1 mm (mean ± standard deviation) preoperatively to < 1.5 mm at last evaluation (p < 0.0001). The median time to syrinx narrowing was 3.6 months following CM-I decompression (95% confidence interval 3.0–6.5 months). After surgery 94% of patients had improved symptoms, but symptoms resolved incompletely in 68% of patients; 52 and 59% of patients had residual dysesthesias and sensory loss, respectively. Clinical improvement occurred before partial or complete disappearance of the syrinx on MR images. Patient age, duration of symptoms, sex, preoperative syrinx diameter, and length of syrinx were unrelated to time to syrinx narrowing. Conclusions Most patients improve after decompression for CM-I, but many have residual symptoms. Syringes may continue to diminish for months to years after surgical decompression. A collapsed syrinx (absence of distention of the spinal cord) indicates that the pathophysiology has been reversed by treatment regardless of the completeness of elimination of the cavity on MR images.


2014 ◽  
Vol 13 (4) ◽  
pp. 368-374 ◽  
Author(s):  
Jakub Godzik ◽  
Michael P. Kelly ◽  
Alireza Radmanesh ◽  
David Kim ◽  
Terrence F. Holekamp ◽  
...  

Object Chiari malformation Type I (CM-I) is a developmental abnormality often associated with a spinal syrinx. Patients with syringomyelia are known to have an increased risk of scoliosis, yet the influence of specific radiographically demonstrated features on the prevalence of scoliosis remains unclear. The primary objective of the present study was to investigate the relationship of maximum syrinx diameter and tonsillar descent to the presence of scoliosis in patients with CM-I–associated syringomyelia. A secondary objective was to explore the role of craniovertebral junction (CVJ) characteristics as additional risk factors for scoliosis. Methods The authors conducted a retrospective review of pediatric patients evaluated for CM-I with syringomyelia at a single institution in the period from 2000 to 2012. Syrinx morphology and CVJ parameters were evaluated with MRI, whereas the presence of scoliosis was determined using standard radiographic criteria. Multiple logistic regression was used to analyze radiological features that were independently associated with scoliosis. Results Ninety-two patients with CM-I and syringomyelia were identified. The mean age was 10.5 ± 5 years. Thirty-five (38%) of 92 patients had spine deformity; 23 (66%) of these 35 patients were referred primarily for deformity, and 12 (34%) were diagnosed with deformity during workup for other symptoms. Multiple regression analysis revealed maximum syrinx diameter > 6 mm (OR 12.1, 95% CI 3.63–40.57, p < 0.001) and moderate (5–12 mm) rather than severe (> 12 mm) tonsillar herniation (OR 7.64, 95% CI 2.3–25.31, p = 0.001) as significant predictors of spine deformity when controlling for age, sex, and syrinx location. Conclusions The current study further elucidates the association between CM-I and spinal deformity by defining specific radiographic characteristics associated with the presence of scoliosis. Specifically, patients presenting with larger maximum syrinx diameters (> 6 mm) have an increased risk of scoliosis.


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